Dermatopathological examination is the “gold standard” for skin lesion diagnosis, which can help to make the most accurate diagnosis of rheumatic immune diseases with skin lesions. The dermatopathological manifestations of skin lesions in most rheumatic diseases are not very specific, while the dermatohistopathological features are relatively distinct in discoid lupus erythematosus, scleroderma, adult Still disease and vasculitis. Dermatopathology plays different roles in the diagnosis of rheumatic immune diseases, differentiation of comorbidities, disease classification and staging, and exploration of pathogenesis of rheumatic diseases. Mastering and applying dermatopathological techniques are very important for the precise diagnosis and treatment of rheumatic diseases.